Von Willebrand disorder

Frequently Asked Questions & Facts - von Willebrand Disorder

What is von Willebrand Disorder (VWD)?
Von Willebrand disorder (VWD) is a bleeding disorder in which people do not have enough of a protein called von Willebrand factor in their blood or the von Willebrand factor does not work properly. Because of these problems, it takes longer for bleeding episodes to stop. VWD is common but not well known, affecting approximately 1% of the population. However, many people will experience few symptoms and many are currently undiagnosed.

VWD affects approximately 200,000 people in Australia. Numbers of those known to have VWD are ever increasing as more people are diagnosed. Most people with VWD have a mild form with few or no symptoms. Unlike haemophilia, von Willebrand disorder occurs in males and females equally.

Symptoms may include frequent nose and gum bleeds, bleeding after dental surgery, easy bruising, heavy menstrual periods or severe bleeding after trauma or surgery. In some cases, bleeding occurs into joints and muscles.

Is von Willebrand disorder only an inherited condition?
There is not always a family history. Some children have VWD because there was a mutation, or change in the gene, during the foetal stage. When this happens the person's chances of passing von Willebrand disorder on to his/her children are the same as those of a parent who has inherited the condition. VWD can also be acquired rather than inherited, but this is very rare.

Can women get VWD?
Yes. Because the VWD gene is linked to an autosome (a chromosome that is not a sex chromosome), not an X or Y chromosome, both males AND females can carry - and be affected by VWD.

What is von Willebrand factor and what does it do?
When blood vessels are damaged, platelets (tiny particles in the blood) clump together at the site of injury; von Willebrand factor is like glue that makes these platelets stick to the areas of blood vessel that are damaged. Von Willebrand factor also acts as a protector of clotting factor VIII. This means some people with von Willebrand disorder may also have a lower amount of factor VIII.

What are the types of von Willebrand disorder?
VWD is divided into "types" according to whether a person has a low amount of von Willebrand factor or has a type of von Willebrand factor that does not work properly, or both. Knowing the type of von Willebrand disorder helps the doctor decide what kind of treatment would be best for that person.

Type 1: This is the most common form of von Willebrand disorder and is usually of mild severity. A person with type 1 von Willebrand disorder has low levels of von Willebrand factor but the von Willebrand factor present functions normally. People with type 1 make up more than 75% of cases of this disorder.Type 2: A person with type 2 often has a normal amount of von Willebrand factor in their blood but has an irregularity in the von Willebrand factor they produce. This type is divided into sub-type 2A, 2B, 2M and 2N. Certain sub-types might require different treatment, which makes knowing the exact type of VWD you have very important.Type 3: A person with type 3 has a total or near total absence of von Willebrand factor. A person with type 3 has severe von Willebrand disorder. He or she will experience regular and/or severe bleeding problems. These could include frequent nose bleeds or heavy periods that require treatment to limit the bleeding. Bleeds into joints and muscles are also common in this type of von Willebrand disorder. People with severe von Willebrand disorder will need treatment before and after any type of surgical or dental procedure. Type 3 is the rarest form of von Willebrand disorder.

How common is VWD?
Figures vary greatly. Some studies indicate it could affect up to 1% of the population. This is one person in 100.

If VWD is so common, why haven't I heard of it?
VWD is usually less severe than haemophilia. For many, it is often so mild that it is not diagnosed at all unless the person has surgery or a major accident.

Why is the condition called VWD?
VWD was discovered in 1925 by, and named after, the Finnish doctor Erik von Willebrand. Professor von Willebrand described this new type of bleeding disorder after observing families living on the Aaland Islands between Sweden and Finland who had bleeding problems. It is also known as von Willebrand disease.

How is VWD different from haemophilia?
The disorder affects both males and females in equal numbers whereas haemophilia tends to mainly affect males. Most people with VWD do not have bleeding symptoms, except during or after surgery or accidents. Those who do bleed, do so mainly from the nose, the intestinal tract and into the skin. People with haemophilia bleed mainly into joints and muscles.

Does everyone carrying the VWD gene have bleeding symptoms?
No. Many people have no symptoms. In fact, they may not even realise they have it. However, the condition can still be passed on to children.

Do I have VWD?
A few bruises do not necessarily indicate the presence of VWD. However, if you think you may have VWD, talk to your doctor about your concerns.

What about surgery?
Surgery, including tooth extractions, can cause bleeding for people with VWD. It is important you tell your surgeon you have VWD well before your surgery date so he/she can work with your haematologist and other key staff to ensure you receive the best possible treatment and care.

Important Note: This information was developed by Haemophilia Foundation Australia for education and information purposes only and does not replace advice from a treating health professional. Always see your health care provider for assessment and advice about your individual health before taking action or relying on published information.

This information may be printed or photocopied for educational purposes.